Open AccessMapt deletion fails to rescue premature lethality in two models of sodium channel epilepsy
Author(s) -
Chen Chunling,
Holth Jerrah K.,
BuntonStasyshyn Rosie,
Anumonwo Charles K.,
Meisler Miriam H.,
Noebels Jeffrey L.,
Isom Lori L.
Publication year - 2018
Publication title -
annals of clinical and translational neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.824
H-Index - 42
ISSN - 2328-9503
DOI - 10.1002/acn3.599
Subject(s) - sodium channel , dravet syndrome , epilepsy , medicine , encephalopathy , tau protein , neuroscience , disease , alzheimer's disease , bioinformatics , biology , psychiatry , sodium , chemistry , organic chemistry
Deletion of Mapt , encoding the microtubule‐binding protein Tau, prevents disease in multiple genetic models of hyperexcitability. To investigate whether the effect of Tau depletion is generalizable across multiple sodium channel gene‐linked models of epilepsy, we examined the Scn1b −/− mouse model of Dravet syndrome, and the Scn8a N1768D/+ model of Early Infantile Epileptic Encephalopathy. Both models display severe seizures and early mortality. We found no prolongation of survival between Scn1b −/− ,Mapt +/+ , Scn1b −/− ,Mapt +/−, or Scn1b −/− ,Mapt −/− mice or between Scn8a N1768D/+ ,Mapt +/+ , Scn8a N1768D/+ ,Mapt +/− , or Scn8a N1768D/+ ,Mapt −/− mice. Thus, the effect of Mapt deletion on mortality in epileptic encephalopathy models is gene specific and provides further mechanistic insight.